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Year : 2020  |  Volume : 21  |  Issue : 2  |  Page : 160-161

Spring cranioplasty in a patient of Apert syndrome: Anesthetic challenges

Department of Anesthesiology and Critical Care, AIIMS, Rishikesh, Uttarakhand, India

Date of Submission28-Dec-2019
Date of Decision29-Jan-2020
Date of Acceptance05-Feb-2020
Date of Web Publication19-Sep-2020

Correspondence Address:
Dr. Sanjay Agarwal
Department of Anesthesia and Critical Care, AIIMS, Rishikesh - 249 203, Uttrakhand
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/TheIAForum.TheIAForum_97_19

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How to cite this article:
Tandon S, Agarwal S, Challa R. Spring cranioplasty in a patient of Apert syndrome: Anesthetic challenges. Indian Anaesth Forum 2020;21:160-1

How to cite this URL:
Tandon S, Agarwal S, Challa R. Spring cranioplasty in a patient of Apert syndrome: Anesthetic challenges. Indian Anaesth Forum [serial online] 2020 [cited 2023 Jun 2];21:160-1. Available from: http://www.theiaforum.org/text.asp?2020/21/2/160/295391


A 6-month-old female child of Apert syndrome, weighing 6.5 kg, was scheduled for spring cranioplasty. She had brachycephaly, hypertelorism, flat nasal bridge, craniosynostosis, bilateral syndactyly in all the four limbs [Figure 1], and acyanotic heart disease with 3 mm ventricular septal defect (VSD) having 60% left ventricular ejection fraction, with left to right shunt. Since the baby was not cooperative, MPS grading could not be assessed. All the investigations were within normal limits. After taking informed written consent from the parents, she was shifted to the operating room, where difficult airway cart was kept ready. Her heart rate was 160/min, blood pressure was 88/45 mmHg, and SpO2 was 100% on room air. Induction of anesthesia was achieved with an increasing concentration of sevoflurane. Two intravenous (iv) cannulas (22G and 24G) were inserted in the saphenous and median cubital vein. Mask ventilation was done using an oral airway. Intubation was done with a 4-mm ID flexometallic endotracheal tube after administering suxamethonium 12.5 mg iv. After prone positioning [Figure 2] and avoiding pressure to the abdomen and eyes, the procedure was started. Perioperative blood loss of around 60–70 ml was replaced. Surgery took almost 4 h followed by an uneventful extubation.
Figure 1: Syndactly

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Figure 2: Position of patient for surgery

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Apert syndrome is a rare autosomal dominant disease affecting fibroblast growth receptor 2, leading to premature fusion of the skull sutures and fusion of digits.[1] Patients also have cardiac abnormalities such as VSD, atrial septal defect, and patent ductus arteriosus and genitourinary problems such as polycystic kidneys, cryptorchidism, and hydronephrosis.[2] Difficult airway in these patients is attributed to the craniofacial abnormalities, posing a threat to the anesthesiologist. Flat nasal bridge makes mask ventilation difficult. Fusion of cervical vertebrae makes it difficult for sniffing position. Deviation of the trachea and tracheal stenosis can lead to difficulty in intubation.[3] Sleeve abnormalities of the trachea lead to accumulation of secretions and can cause bronchospasm.[4] Therefore, difficult airway cart should be kept prepared. Tracheostomy is not a good option if cannot intubate cannot ventilate situation arises due to tracheal abnormalities and percutaneous cricothyroidotomy is less likely required in such children with facial dysmorphism.[5] They are prone to hypothermia as these patients tend to sweat a lot. The child was kept warm by giving warm fluids and wrapped by cotton on the back and limbs. Venous air embolism (VAE) is another dreadful complication in these cases which can occur during cranioplasty. Continuous monitoring of EtCO2 is essential to diagnose VAE. A drop >5 mmHg of EtCO2 suggests the possibility of VAE. A thorough preoperative evaluation and preparation is essential for the management of such patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Chen L, Li D, Li C, Engel A, Deng CX. A Ser252Trp [corrected] substitution in mouse fibroblast growth factor receptor 2 (Fgfr2) results in craniosynostosis. Bone 2003;33:169-78.  Back to cited text no. 1
Palmisango BW. Anaesthesia for plastic surgery. In: Gregory GA, editor. Pediatric Anaesthesia. 3rd ed. New York: Churchill Livingstone; 1994. p. 699-740.  Back to cited text no. 2
Morris GP, Cooper MG. Difficult tracheal intubation following midface distraction surgery. Paediatr Anaesth 2000;10:99-102.  Back to cited text no. 3
Basar H, Buyukkocak U, Kaymak C, Akpinar S, Sert O, Vargel I. An intraoperative unexpected respiratory problem in a patient with Apert syndrome. Minerva Anestesiol 2007;73:603-6.  Back to cited text no. 4
Perkins JA, Sie KC, Milczuk H, Richardson MA. Airway management in children with craniofacial anomalies. Cleft Palate Craniofac J 1997;34:135-40.  Back to cited text no. 5


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